Idiopathic Portal Hypertension

Abstract

A new type of portal hypertension was observed from the clinical stydy of all patients referred for portacaval shunt surgery. The disease was characterized by splenomegaly and recurrent hematemesis starting 3 to 6 yrs. prior to study. The physical examination revealed only splenomegaly. Portal pressure in both the spleen and wedged hepatic venous positions was elevated, and the total blood flow to the liver was normal. The liver function tests were also normal with the exception of sulfobromopthalein retention. Histology shows fibrosis limited to the small portal veins and occasionally occlusion of the lumen of medium size portal veins. Splenic portal venography shows marked narrowing of the intermediate portal veins. These patients seem a distinct group. Comparison with descriptions of similar disease throughout the world and a theroy of the etiology is presented.